Objective: To describe the clinical features of anti-myelin oligodendrocyte glycoprotein (MOG) antibody-positive cortical encephalitis in children. Methods: Patients who were hospitalized in Beijing Children's Hospital from June 2018 to October 2019, with positive MOG antibodies and phenotype of cortical encephalitis were retrospectively analyzed. Cell-based assays (CBAs) were used to test MOG antibodies. Results: Five patients had the phenotype of cortical encephalitis during follow-up, with 3 females and 2 males. The age of onset ranged from 8 years to 12 years and 1 month. At the last follow-up, 3 cases exhibited a monophasic course and 2 cases were with relapse and remission courses. Six out of 8 episodes which had the phenotype of cortical encephalitis presented with seizures, among which 3 episodes had status epilepticus. None had recurrent seizures during remission. Other symptoms included fever (7/8), headache and vomiting (4/8), somnolence (3/8) and hemiplegia (1/8). Unilateral cortical swelling was observed in cerebral magnetic resonance imaging (MRI) of all patients, without any hemorrhage and necrosis. White blood cell (WBC) counts of cerebrospinal fluid increased, ranging from8×10(6)/L to 186×10(6)/L. All patients recovered well after treatment with intravenous immunogloblin and glucocorticoid. Two patients had relapses during follow-up and were additionally treated with mycophenolate mofetil. Conclusions: Anti-MOG antibodies can induce cortical encephalitis. In clinical setting, fever, headache and seizures are common, however, severe consciousness disturbance and local neurological deficits are rare in these patients. Cerebral MRI shows unilateral cortical swelling without any hemorrhage and necrosis. Usually, immunotherapy works well. No patients exist repeated seizures in remission, but some patients may have relapses.
目的： 描述儿童抗髓鞘少突细胞糖蛋白（MOG）抗体阳性皮质脑炎临床特点。 方法： 回顾性分析2018年6月至2019年10月于北京儿童医院神经内科住院，血清抗MOG抗体检测阳性，且病程中存在皮质脑炎表型患儿的临床特点。抗MOG抗体检测使用基于转染细胞（CBA）的间接免疫荧光法。 结果： 共5例患儿病程中存在1次及以上皮质脑炎发作，女3例，男2例。起病年龄8岁至12岁1个月。至随访末3例为单相病程，2例为复发缓解病程。8次以皮质脑炎为表型的发作中，6次有癫痫发作，其中3次为癫痫持续状态，缓解期患儿无遗留反复癫痫发作。其他症状包括：发热（7/8），头痛、呕吐（4/8），嗜睡（3/8），偏瘫（1/8）。头颅MRI均存在单侧皮质肿胀，病灶无出血坏死。脑脊液白细胞存在不同程度升高（8×10(6)/L~186×10(6)/L）。所有患儿急性期使用丙种球蛋白及糖皮质激素治疗后症状均消失，其中2例有复发，加用吗替麦考酚酯口服。 结论： 抗MOG抗体可介导皮质脑炎表型，临床以发热、头痛、癫痫发作常见，严重意识障碍及局灶神经功能受累少见；头颅MRI常表现为单侧皮质肿胀，无出血坏死；免疫治疗大多效果好，缓解期无遗留癫痫发作，部分患者可复发。.
Keywords: Anti-myelin oligodendrocyte glycoprotein antibody; Encephalitis.